Pulmonary Involvement in Systemic Autoimmune Diseases, Volume 2 (Handbook of Systemic Autoimmune Diseases)

297 Pages·2004·5.58 MB·English

by  Andrea Doria

Handbook of
Systemic Autoimmune Diseases
Volume 2
Pulmonary Involvement in Systemic
Autoimmune Diseases
Handbook of
Systemic Autoimmune Diseases
SeriesEditor:Ronald A. Asherson
Volume 1 The Heart in Systemic Autoimmune Diseases
Edited by: A. Doria and P. Pauletto
Volume 2 Pulmonary Involvement in Systemic Autoimmune Diseases
Edited by: A.U. Wells and C.P. Denton
Handbook of
Systemic Autoimmune Diseases
Volume 2
Pulmonary Involvement in Systemic
Autoimmune Diseases
Edited by:
Athol U. Wells
Royal Brompton Hospital
Chelsea, London, United Kingdom
Christopher P. Denton
Royal Free Hospital, Centre of Rheumatology
London, United Kingdom
Series Editor
Ronald A. Asherson
2004
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Preface
Pulmonarycomplicationsarenowthemostfrequentcauseofdeathinmanysystemicautoimmunedisordersand
areamajorandincreasingsourceofmorbidity.Inpart,thismayreflectbetteroutcomesinrecentdecadeswiththe
development ofoptimalimmunosuppressive protocols; patients now survive long enough todevelop pulmonary
problems. Thus, there is now a particular need for pulmonary evaluation to be accurate and pragmatic, whether
undertakenbyrheumatologistsorchestphysicians.However,publishedreviewshavetendedtofocusuponsingle
systemicautoimmunedisorders,ratherthanprovidingdetailedoverviewsofpulmonaryinvolvementinsystemic
autoimmune disease at large. We were delighted to accept the invitation from Ronald Asherson to redress this
deficiency.Ourfellow-contributors,allleadingclinicalscientistsinvariousspecialities,wereequallyenthusiastic.
Theresultingmonographcoversthefieldfromanumberofangles,withparticularemphasisonfourbroadareas.
Thefirst twochapters aredevotedtothe range oflung morphological abnormalities insystemicautoimmune
diseases.Histologicalevaluationhaslongbeenregardedasthediagnosticcornerstoneindiffuselungdisease,and
recent advances in the field, notably the definition of non-specific interstitial pneumonia, have been particularly
relevant to our readership. High resolution computed tomography of the lungs has revolutionised the routine
clinical assessment of systemic autoimmune disease, by distinguishing between a bewildering array of possible
pulmonary complications, and serving as an invaluable prognostic guide. Optimal management requires that
clinicians make the best use of both disciplines, with appropriate recourse to surgical biopsy when clinical and
imagingfindings are inconclusive.
The next part consists of three chapters on pulmonary vascular disorders. There is a general overview of
pulmonary hypertension in connective tissue disease, with Wegener’s granulomatosis and other vasculitides
coveredseparately.Thedetailcontainedwithinthissectionreflectsourviewthatthevasculardiseasesarethemost
difficultofallpulmonarycomplicationstodetectandmanage,especiallywhentherearecoexistingabnormalities
inthe lung interstitium.
Sevenchaptersaregroupedinwhichthepulmonarycomplicationsofavarietyofindividualsystemicdisorders
arereviewed.Thesedisordersarenotableforawidevarietyofpulmonarydiseaseprocesses,withthevariableco-
existence of pulmonary interstitial, pulmonary vascular and extra-pulmonic restrictive abnormalities. Accurate
clinical evaluation is critically dependent upon the knowledge of the profile of each individual disease, without
whichlogicalinvestigationandfocusedmanagementisunattainable.Thechaptersinthissectionarewrittenwith
the practising clinicianin mindand can beapplied equally by rheumatologists andchest physicians.
Finally,theeditorswouldliketohighlightthechapterondruginducedlungdiseasebyPhilippeCamus.Withan
exponentialexpansionintreatmentsusedinsystemicautoimmunedisease,iatrogenicdiseasehasbecomeanissue
ofconcern.Despiteanincreasinglyurgentneedtoprovideguidanceforclinicians,thetopichasnotpreviouslybeen
dealtwithdefinitively.WeareproudtoofferthereviewbyCamusasareferencestatementinthisdifficultarea.
Athol U. Wells
Christopher P. Denton
v
Series Editor
Dr RonaldA. Asherson
DrRonaldA.Asherson,MD.FACP,MD(Hon)(London),FCP(SA),FACR,isHonoraryConsultantPhysicianat
theRheumaticDiseaseUnit,DepartmentofMedicine,UniversityofCapeTownHealthSciencesCentreinCape
Town,aswellasbeingaConsultantRheumatologistattheRosebankClinicinJohannesburg,SouthAfrica.Heis
also a Visiting Professor at the Systemic Autoimmune Diseases Unit at the Hospital Clinic, Barcelona, Spain
where heregularlyvisitsand coordinates researchprojects.
DrAshersonqualifiedinMedicineattheUniversityofCapeTownin1957and,aftercompletinghisinternship,
becameH/PtoProf.SirChristopherBoothattheHammersmithHospital,London,in1960.In1961heaccepteda
FellowshipattheColumbiaPresbyterianHospitalinNewYork,returningin1962tobecomeRegistrarandthen
Senior Registrar at Groote Schuur Hospital in Cape Town in 1964. After 10 years as a Clinical Tutor in the
Department of Medicine, he returned to the United States and was appointed as Assistant Clinical Professor of
MedicineattheNewYorkHospital–CornellMedicalCentreunderthelateProfessorHenryHeineman.From1981
to1986,hewasassociatedwiththeRheumatologyDepartmentattheRoyalPostgraduateMedicalSchoolofLondon.
ItwasatthattimethathedevelopedhisinterestinConnectiveTissueDiseasesandAntiphospholipidAntibodies.
In1986hemovedtotheRayneInstituteandStThomas’HospitalinLondon,wherehewasappointedHonorary
ConsultantPhysicianandSeniorResearch Fellow.In1991hetook asabbaticalatStLuke’sRooseveltHospital
CentreinNewYork,workingwithProf.RobertLahita.In1992hereturnedtoSouthAfricatoprivatepracticein
Johannesburg.
In1998hewaselectedasFellowoftheAmericanCollegeofPhysicians(FACP)aswellasaFoundingFellow
of the American College of Rheumatology (FACR). From 1988 to 1991 he served on the Council of the Royal
SocietyofMedicineinLondon.In1992hewasco-winneroftheEuropeanLeagueAgainstRheumatism(EULAR)
Prizeandin1993wastheco-recipientoftheInternationalLeagueAgainstRheumatism(ILAR)Prize,bothforhis
research on antiphospholipid antibodies.In 1994 he was elected as a Fellow of the Royal College ofPhysicians
(FRCP)ofLondon.In2002hewasawardedanHonoraryDoctorateinMedicinefromtheUniversityofPlevenin
Bulgaria.
DrAshersonhasbeenaninvitedspeaker atmanyuniversitiesandInternationalconferences bothintheUSA
andinEurope.Heistheauthorofmorethan280papersonconnectivetissuediseasesandhascontributedtomore
than30textbooksofmedicine,rheumatologyandsurgeryaswellashavingco-edited“ProblemsintheRheumatic
Diseases”, the “Phospholipid Binding Antibodies”, two editions of “The Antiphospholipid Syndrome” and
“Vascular Manifestations of the Systemic Autoimmune Diseases”. He is currently engaged in research on
connective tissue diseases, particularly on the antiphospholipid syndrome together with colleagues in the USA,
Spain,FranceandIsraelmainlyandisinclinicalpracticeinSouthAfrica.In1999hewastheco-recipientofthe
Juan Vivancos Prize in Spain and in 2003 was the co-recipient of the Abbott Prize, awarded at the European
League Against Rheumatism (EULAR) International Meeting, held inLisbon, Portugal.
Hisoriginaldescriptionofthe“CatastrophicAntiphospholipidSyndrome”andthepublishingofmorethan40
papers on this new disease was rewarded by the attachment of the eponym “Asherson’s Syndrome” to this
conditionattheNovember2002InternationalPhospholipidConferenceheldinSicily.Hehasestablishedthefirst
International Committeeto study survivorsof thisSyndrome.
He is currently editing a series of 12 volumes entitled “The Handbook of Systemic Autoimmune Disease”
(Elsevier,TheNetherlands)andinSeptemberof2003wasCo-ChairmanoftheFirstLatinAmericanCongresson
Autoimmunity,heldintheGalapagosIslands,Ecuador.Heco-chairedandparticipatedinaSessionattheMilan
Conference on “Heart, Rheumatism andAutoimmunity” held inFebruary 2004.
He was appointed to the International Advisory Board of the International Conference on Systemic Lupus
Erythematosus held inNew York inMay of2004 and also participated inthis Meeting.
vii
Volume Editors
Athol U. Wells
Athol Wells graduated in Medicine at the University of Otago in 1979 and turned to respiratory medicine after
completingtrainingasageneralphysician.From1986to1989,hewasarespiratorytutorspecialistatGreenLane
Hospital, in Auckland, New Zealand, and then moved to the Royal Brompton Hospital in London as a research
fellow,initially.AfterreturningtoNewZealandinthemid1990s,hesubsequentlyacceptedanappointmentasa
consultant chest physician at the Royal Brompton Hospital and is now one of two chest physicians who run the
interstitial lung disease unit at that institution. He, therefore, has a 15-year expertise in the evaluation and
management of the pulmonary complications of connective tissue diseases. The unit in which he works is
recognised internationally as a leading world centre for lung disease in systemic autoimmune disease. His own
personal research has focused on clinical science, with particular reference to prognostic evaluation,
structure/function relationships and CT evaluation of interstitial lung disease at large and, especially, lung
diseaseinsystemicsclerosis.Morerecently,hehasappliedhisexpertisetothedefinitionofphenotypeingenetic
studies. He is the author of numerous clinical studies and is widely involved in post-graduate education as a
speakerand writer of review articlesand book chapters.
Christopher P. Denton
Christopher Denton graduatedin Medicine fromGuy’s Hospital in London in 1986 and undertook postgraduate
training in internal medicine and rheumatology at Northwick Park, St. Thomas’ and the Royal Free Hospital in
London. He obtained a PhD invascular biology at University College London in1997and gained his specialist
accreditationinrheumatologyin1998.Afterfurtherlaboratoryandclinicaltrainingingeneticsandrheumatology
as a Wellcome Advanced Fellow at the Texas Medical Centre in Houston, he returned to the UK to join the
CentreforRheumatologyattheRoyalFreeHospitalinLondon,supportedbyaSeniorResearchFellowshipfrom
the Arthritis Research Campaign(UK).
Heiscurrentlyaseniorlecturerinrheumatologyandconsultantrheumatologistinthesclerodermaclinicatthe
RoyalFreewhichreviewsmorethan1000patientswithsystemicsclerosisandrelateddisorders.Hehasextensive
clinical and laboratory research interests in the field of scleroderma and works closely with cardiologists and
respiratory physicians at the Royal Free and the Royal Brompton Hospitals to investigate and treat pulmonary
complications of connective tissue disease. In 2003 Christopher Denton was elected as a Fellow of the Royal
College ofPhysicians inLondon.
ix
List of Contributors
Ronald A. Asherson Joel David
Rheumatic Diseases Unit Department ofRheumatology
Departmentof Medicine Nuffield Orthopaedic Centre
Universityof CapetownHealth Sciences Centre Oxford OX3 7LD
Groote SchuurHospital UK
Capetown [email protected]
South Africa
Garie Davies
Alexander N.Bennett
Royal Brompton Hospital
The Lupus Research Unit
Chelsea London
The Rayne Institute
UK
StThomas’ Hospital
London SE1 7EH
David P. D’Cruz
UK
TheLupusResearchUnit
CarolM.Black TheRayneInstitute
Centrefor Rheumatology StThomas’Hospital
Royal Free Hospital London SE1 7EH
London, NW32QG UK
UK david.d’[email protected]
Roland M. du Bois
JaneDeng
Interstitial Lung DiseaseUnit
Division of Pulmonaryand
Royal Brompton Hospital
Critical Care Medicine
London, SW3 6NP
Department ofInternalMedicine
UK
UniversityofMichigan Medical Center
[email protected]
Ann Arbor, MI
Philippe Camus USA
Departmentof Pulmonaryand IntensiveCare
UniversityMedical Center Le Bocage ChristopherP. Denton
and Medical School Centre for Rheumatology
Universite´ de Bourgogne Royal Free Hospital
Dijon London, NW3 2QG
France UK
[email protected] [email protected]
Ricard Cervera Sally Edmonds
SystemicAutoimmune Diseases Unit Department ofRheumatology
Hospital Cl´ınic Nuffield Orthopaedic Centre
Barcelona Catalonia Oxford OX3 7LD
Spain UK
xi
xii ListofContributors
Gerard Espinosa Michael P. Keane
SystemicAutoimmune Diseases Unit Division of Pulmonary, Critical Care Medicine and
Hospital Cl´ınic Hospitalists
Barcelona David Geffen School ofMedicine at UCLA
Catalonia LosAngeles, CA
Spain USA
Josep Font Peter Lamprecht
SystemicAutoimmune Diseases Unit Department ofRheumatology
Hospital Cl´inic UniversityHospital ofSchleswig-Holstein
Barcelona Catalonia Luebeck
Spain Germany
Toma´s Franquet Rheumaklinik Bad Bramstedt
Department of DiagnosticRadiology RatzeburgerAllee160 D-23538
Hospital deSant Pau Luebeck
Universitat Auto´noma de Barcelona Germany
Barcelona
Spain Joseph P.Lynch III
[email protected] Division of Pulmonary, Critical Care Medicine
andHospitalists
Mario Garc´ıa-Carrasco
David Geffen School ofMedicine at UCLA
SystemicAutoimmune Diseases Unit
LosAngeles, CA
Hospital Cl´ınic
USA
Barcelona
[email protected]
Catalonia
Spain Susan Manzi
Division of Rheumatology and Clinical Immunology
NicoleS. Goh
UniversityofPittsburgh School ofMedicine
Interstitial Lung DiseaseUnit
andUniversity ofPittsburghGraduateSchool
Royal Brompton Hospital
ofPublic Health
London, SW3 6NP
Pittsburgh, PA
UK
USA
WolfgangL. Gross [email protected]
Department of Rheumatology
HaralamposM. Moutsopoulos
UniversityHospital ofSchleswig-Holstein
Department ofPathophysiology
Luebeck
Laiko Hospital
Germany
School ofMedicine
Rheumaklinik Bad Bramstedt National and Kapodistrian Universityof Athens
Ratzeburger Allee 160 Athens
D-23538 Luebeck Greece
Germany [email protected]
[email protected]
Andrew G.Nicholson
Amy H.Kao Department ofHistopathology
DivisionofRheumatology and Clinical Immunology Royal Brompton Hospital
Universityof PittsburghSchool of Medicine London, SW3 6NP
Pittsburgh,PA UK
USA [email protected]